title: Heterotaxy syndrome and intestinal rotation abnormalities: a survey of institutional practice.
abstract: OBJECTIVE
  Abnormalities of intestinal rotation (IRA) are commonly associated with heterotaxy syndrome (HS). There is controversy whether asymptomatic infants with HS require screening for IRA and if present, whether a prophylactic Ladd procedure is indicated. The objective of this study is to determine institutional practice across North America in the management of asymptomatic infants with HS and IRA.
  
  
  METHODS
  We performed an international, multi-institutional web based survey to examine current practice and opinions in the management of IRA in HS patients.
  
  
  RESULTS
  Overall response rate was 30%. Of physicians surveyed, 84% believe that HS patients should be screened for IRA in the neonatal period. 61% of general surgeons, 50% of cardiovascular surgeons and 45% of cardiologists feel that all patients with HS and an asymptomatic IRA should have a prophylactic Ladd procedure. 55% of physicians stated they would be comfortable with conservative management for patients with HS and asymptomatic IRA.
  
  
  CONCLUSIONS
  The risk of midgut volvulus, morbidity and mortality from elective procedures and cardiovascular prognosis must be considered prior to an elective Ladd procedure on asymptomatic HS patients. There are practice variance among sub-specialists caring for these patients, a lack of expert consensus, and a paucity of evidence-based data for IRA in this population.
authors.affiliation: Department of Pediatrics, University of Alberta, Edmonton, AB, Canada.
authors.forename: Charissa R
authors.initials: CR
authors.lastname: Pockett
authors.forename: Bryan J
authors.initials: BJ
authors.lastname: Dicken
authors.forename: Ivan M
authors.initials: IM
authors.lastname: Rebeyka
authors.forename: David B
authors.initials: DB
authors.lastname: Ross
authors.forename: Lindsay M
authors.initials: LM
authors.lastname: Ryerson
mesh_terms: D001291:Attitude of Health Personnel; D002309:Cardiology; D004065:Digestive System Abnormalities / Q000175:diagnosis / Q000601:surgery; D019538:Health Care Surveys; D059446:Heterotaxy Syndrome / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007325:Institutional Practice; D045822:Intestinal Volvulus / Q000175:diagnosis / Q000601:surgery; D009656:North America; D010372:Pediatrics; D010818:Practice Patterns, Physicians' / Q000706:statistics & numerical data*
keywords: HS; Heterotaxy Syndrome; Heterotaxy syndrome; IRA; Intestinal Rotation Abnormality; Isomerism; Ladd procedure; Malrotation